Diabetes, Heart Disease, & Stroke - NIDDK
What Is Pulmonary Hypertension?
Pulmonary hypertension (PH) is a condition that occurs when your blood pressure in the blood vessels between the heart and lungs is abnormally high. Blood pressure measures the amount of force it takes to push your blood through your arteries, which routes blood to the rest of your body. With pulmonary hypertension, the heart has to work harder to carry blood from the heart to the lungs, and symptoms such as shortness of breath, fatigue, chest pain, and lightheadedness can develop.
Pulmonary hypertension affects about 1% of the global population. Some cases of pulmonary hypertension are hereditary, and others develop due to underlying medical conditions. Pulmonary hypertension is a serious, progressive condition that requires treatment, such as medication, lifestyle modifications, oxygen therapy, or surgery.
The five types of pulmonary hypertension are classified based on the underlying cause. Treatment options can vary depending on the type of pulmonary hypertension you have. Group 1: Pulmonary Arterial Hypertension (PAH) Pulmonary arterial hypertension causes the small lung arteries to become narrow, stiff, or thickened. PAH is often idiopathic, meaning there is no identified cause. Drug use, congenital heart disease, HIV, connective tissue disorders, and autoimmune diseases can also cause this type. Group 2: Pulmonary Hypertension Due to Left Heart Disease The most common form of PH, this type is a complication of heart conditions that affect the left side of the heart or heart valves, causing your heart to struggle with pumping blood returning from the lungs. Group 3: Pulmonary Hypertension Due to Lung Disease This type causes the lung arteries to constrict (tighten) so that blood only reaches areas of the lungs that receive the most oxygen, leading to increased blood pressure in the lungs. This can occur due to a variety of factors, including living in high altitudes, having sleep apnea, or having a lung disorder like chronic obstructive pulmonary disease (COPD) or interstitial lung disease. Group 4: Pulmonary Hypertension Due To Chronic Blood Clots in the Lungs Also known as chronic thromboembolic pulmonary hypertension (CTEPH), this type is a rare, progressive form of pulmonary hypertension that develops when blood clots frequently form in the pulmonary arteries, causing narrowed or clogged arteries. Group 5: Pulmonary Hypertension With Unknown Causes Some cases of pulmonary hypertension don't fit neatly into the first four categories. Certain conditions, such as sickle cell anemia, certain metabolic disorders, and sarcoidosis, can cause this fifth type. In the initial stages, symptoms of pulmonary hypertension are often mild or similar to symptoms of other conditions. As the disease progresses, symptoms may worsen and include: Shortness of breath, especially during physical activity Fatigue Weakness Dry cough (sometimes with blood) Dizziness or fainting Nausea Vomiting Hoarse voice Swelling in the abdomen, ankles, legs, or feet Chest pain or discomfort, especially during physical exertion Wheezing (whistling sound when breathing) Rapid heartbeat Decreased appetite Upper right abdominal pain Stages The World Health Organization classifies the four stages of pulmonary hypertension based on the severity of symptoms. They include: Class I: You have no limitations in physical activity; ordinary activities do not cause symptoms. Class 2: You have slight limitations in physical activity and are comfortable at rest, but ordinary physical activity causes symptoms. Class 3: You have significant limitations in physical activity and may experience symptoms during ordinary physical activity but not at rest. Class 4: You cannot perform any physical activity without symptoms and may also have symptoms when resting. Pulmonary hypertension develops when the arteries in your lungs narrow, stiffen, or become damaged, making your heart work harder to pump blood through your lungs, where it picks up oxygen. At rest, normal pulmonary blood pressure is between 11-20 mm Hg (millimeters of mercury/pressure measurement). If your pulmonary blood pressure is 25 mm Hg or higher, you have pulmonary hypertension. Sometimes, there is no identifiable cause of pulmonary hypertension; other times, it may develop due to an inherited (genetic) condition or underlying heart condition. Underlying health conditions that can affect or damage the blood vessels can cause pulmonary hypertension. These include: Respiratory diseases: Conditions that cause breathing problems, including obstructive sleep apnea, COPD (e.G., emphysema, chronic bronchitis), and pulmonary fibrosis, can affect the heart and blood vessels. Connective tissue diseases: Scleroderma, lupus, and mixed connective tissue disease can inflame and damage blood vessels, leading to pulmonary hypertension. Heart disease: Heart problems that affect the left side of your heart, such as coronary artery disease, valvular heart disease, and congenital heart defects, can restrict blood flow to the lungs. Blood clots: Pulmonary embolism, or a blood clot in the lungs, can block one of the pulmonary arteries and lead to pulmonary hypertension. Other conditions: HIV, liver disease, infections, and certain drugs (e.G., methamphetamine and cocaine) can damage blood vessels and cause pulmonary hypertension. Factors that may increase your risk of pulmonary hypertension include: Age: The risk of pulmonary hypertension increases with age. Most people receive a diagnosis between the ages of 30 to 60. Sex: Pulmonary hypertension is more common in people assigned female at birth than people assigned male at birth. Family history: A family history of blood clots or pulmonary hypertension increases your risk of developing the condition. Medications: Some prescription medications, such as appetite suppressants, opioids, selective serotonin reuptake inhibitors (SSRIs), and some drugs used to treat cancer are associated with an increased risk of pulmonary hypertension. Lifestyle habits: Certain lifestyle habits, such as illicit drug use or smoking, raise the risk of pulmonary hypertension. Environment: Living at a high altitude for an extended period, parasitic infections, or exposure to asbestos all increase your risk. Diagnosis of pulmonary hypertension involves a medical history review, physical examination, and diagnostic testing. To diagnose pulmonary hypertension, your healthcare provider will ask about your symptoms, current medications, and your personal and family medical history. During the physical examination, they will listen to your heart and lungs, measure your blood pressure, and check your legs, abdomen, and feet for signs of fluid buildup (edema). Depending on your symptoms and risk factors, your healthcare provider may refer you to a cardiologist (a doctor specializing in heart and blood vessel diseases) or pulmonologist (a doctor specializing in lung diseases) who will order diagnostic tests to determine if you have pulmonary hypertension. These tests may include: Blood tests: These can be used to check for anemia, blood clots, or cardiac stress. Cardiac imaging scans: Cardiac magnetic resonance imaging (MRI) provides detailed images of the heart and surrounding blood vessels so your healthcare provider can view their structure and assess their level of function. Lung imaging scans: Imaging tests, such as a chest X-ray, take detailed pictures of the lungs and surrounding structures to help visualize the shape of the heart and pulmonary arteries. Electrocardiogram (ECG/EKG): This measures the electrical activity of your heart to detect damage to your heart or surrounding blood vessels. It can determine if your heart is working too hard to pump blood to the lungs. There is no cure for pulmonary hypertension, but treatments can help manage symptoms and slow disease progression. Treatment options include medications, oxygen therapy, various procedures, and lifestyle modifications. Medications Prescription drugs can help lower your blood pressure in the pulmonary arteries, improve blood flow to the lungs, and reduce inflammation. Oral medications (taken by mouth) commonly prescribed for pulmonary hypertension include: Vasodilators: These medications help relax your blood vessels to reduce pressure in your pulmonary arteries. Examples include calcium channel blockers, such as Adizem (diltiazem) and Adalat (nifedipine). Endothelin receptor antagonists: These block the effects of endothelin, a protein that constricts and narrows blood vessels. Examples include Letairis (ambrisentan), Tracleer (bosentan), and Opsumit (macitentan). Phosphodiesterase 5 inhibitors: These help relax blood vessels to improve blood flow and reduce blood pressure. Examples include Revatio (sildenafil) and Adcirca (tadalafil). Pulmonary hypertension medications are sometimes delivered subcutaneously (with a small needle under the skin), intravenously (IV), or breathed in through an inhaler. These medicines help open the blood vessels to manage symptoms such as shortness of breath and chest pain. These include: Remodulin, Tyvaso (treprostinil) Flolan, Veletri (epoprostenol) Ventavis (iloprost) Oxygen Therapy Supplemental oxygen may be necessary to improve your oxygen levels and reduce symptoms of pulmonary hypertension, such as shortness of breath. Oxygen therapy is helpful for more advanced cases of PH where shortness of breath occurs during physical activity or at rest. Specialist Procedures In some cases, surgery or other procedures may be necessary to treat pulmonary hypertension. These include: Balloon atrial septostomy: This procedures involves widening the hole (foramen ovale) between the left and right atriums of your heart to improve blood flow and increase blood oxygen levels. Balloon pulmonary angioplasty: This procedure opens blood vessels in your lungs that are narrow or blocked. This reduces blood pressure in the blood vessels of your lungs and improves your heart function. Pulmonary endarterectomy: This surgery removes old blood clots and scarring inside pulmonary blood vessels. It may cure some cases of group 4/pulmonary hypertension due to chronic blood clots. Lifestyle Modifications Along with medical treatments, healthy lifestyle can help manage symptoms of pulmonary hypertension. Heart-healthy lifestyle habits include: Exercise: Regular physical activity, such as walking, swimming, or cycling, benefits cardiovascular health. Avoid heavy lifting or strenuous exercise that causes chest pain or worsens your symptoms. Healthy diet: A nutritious, balanced diet with plenty of fruits, vegetables, whole grains, lean proteins, and low-fat dairy products may help slow disease progression. Limit your intake of fat, cholesterol, sodium (salt), and processed sugars. Avoid smoking: If you smoke, talk to your healthcare provider about smoking cessation programs to help you quit. Stress management: Living with pulmonary hypertension can take a toll on your emotional and psychological well-being. Finding healthy ways to manage stress can help you cope with the stressors of PH. It is not always possible to prevent pulmonary hypertension, but there are things you can do to reduce your risk. This includes seeing your healthcare provider regularly for checkups so they can help detect symptoms of PH early. This is especially true if you have a medical condition associated with an increased risk of PH, such as COPD or sleep apnea. Following your treatment plan for any chronic conditions you have can help prevent PH, Regular physical activity, consuming a nutritious diet, and maintaining a healthy weight are important for your overall health, and especially your heart. If you smoke, quitting is one of the best ways to protect your heart, blood vessels, and lungs. Pulmonary hypertension is a progressive disease that can worsen over time. People with PH are at an increased risk of developing other conditions, including: Anemia: Develops when the body does not have enough healthy red blood cells. PH can cause anemia if the bone marrow, where red blood cells are produced, does not get sufficient oxygen. PH can also increase the breakdown of red blood cells and lead to anemia. Arrhythmia: High blood pressure in the pulmonary blood vessels can lead to heartbeat irregularities. Blood clots: Narrowed blood vessels can slow blood flow, causing it to clump together and form clots within the blood vessels. Heart failure: Right-sided heart failure occurs when the heart cannot pump enough blood to meet your body's needs. Pericardial effusion: Increased blood pressure in the pulmonary vessels can cause fluid buildup around your heart. Pulmonary hypertension is a chronic condition that can impact every part of your life. With proper management, most people with PH can control their symptoms and live well. Along with working closely with your healthcare team, being proactive about your health can help slow disease progression. This includes following your treatment plan (including taking medications as prescribed) and having regular check-ups with your healthcare provider. It is safest to avoid activities that may worsen your symptoms, including intense workouts, lifting heavy objects, soaking in a hot tub, and spending prolonged periods at high altitudes.Thanks for your feedback!
From Continuous Doctors Appointments To Open Heart Surgery, One Baby Is Defying The Odds
From continuous doctors appointments to open heart surgery, one baby is defying the odds
{p}Little KJ had open heart surgery when he was just eight months and he still faces more surgeries (WACH/ KEI'YONA JORDON){/p}
Columbia, S.C (WACH) —
A little boy in the Midlands is beating the odds every day.
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Little KJ had open heart surgery when he was just eight months and he still faces more surgeries.
Read MoreHealth experts say one out of every 33 children are born with birth defects in South Carolina and those defects are the leading cause of infant mortality.
Kristen Moore said she still has nightmares after watching her son KJ Moore undergo open heart surgery.
KJ was diagnosed with the rare heart defect Aortopulmonary Window which only affects one percent of babies.
It led to other life-threatening issues including permanent lung failure. KJ struggled with illness after he was born and was only strong enough to have surgery to fix the issue when he was eight months old.
"I still have dreams and nightmares," said Moore. "When you see your baby and the chest is open and then there are wires."
Moore said before KJ was born doctors said her son would be born with a hole in his heart, but things got worse after doctors noticed the umbilical cord was wrapped around his neck and they were forced to deliver him prematurely.
"When I woke up his dad was in the hospital and he was like I need to tell you something before I hand him to you," shared Moore. "KJ doesn't have thumbs and KJ's arms are turned in."
Joyce Kimrey is the Director Of Early Intervention at the South Carolina Department of Disabilities and Special Needs.
She explained there is a wide range of services for families with children who have birth defects.
"There are hundreds of, if not thousands of birth defects," said Kimery. "Some of the more common ones are spine bifida, heart defects, cleft lip, and cleft pallet."
One of them is called Baby Net, which offers different types of therapies and rehabilitation free of charge.
"It's important to let mothers know we are with them and that they have help," stated Kimrey.
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Open Heart Surgeries Rescue Children With Heart Defects
At Mater Hospital in Nairobi, 23-year-old Lucyline Kinya from Isiolo has been here for days, waiting for her daughter to undergo a heart surgery.
It all began when her daughter, who was six months old, started having chest problems and was diagnosed with pneumonia.
"When she was one year and two months I changed the hospital we were going to and went to Isiolo Referral Hospital where they ordered an X-ray that showed she had an enlarged heart. The hospital made another request for an echo test to be done," says Lucyline.
An echocardiogram, or "echo", is a type of ultrasound scan used to look at the functioning of the heart and nearby blood vessels.
The results showed her daughter had a hole in the heart, and the only treatment was to do open heart surgery as its effective in treating atrial septal defects.
"We didn't have the 1.5 million that was needed to perform the surgery. And she had to wait until this year when her daughter was scheduled for surgery," Lucy says.
She says it has been a long wait and that meanwhile she has had to take her daughter to the hospital twice.Sometimes she can't breathe she has to be put on oxygen.
"When I was told she has a hole in her heart, I almost fainted and wondered why God did this to me. I am even afraid to give birth to another child because of what my daughter hasgoe through," she explains.
She is not the only one going through the agony. Clementino Obeli from Nambale in Busia County has had a similar struggle with the misdiagnosis of her grandson's condition.
We spoke to her while her grandson was undergoing open heart surgery at Mater Misericordiae Hospital.
Open-heart surgery is any type of surgery where the chest is cut open and surgery is performed on the muscles, valves, or arteries of the heart.
"When the baby was brought to me, he could not sit only squat and had to put fingers in his mouth to breathe. Some told me the baby will die or be bewitched but I never give up," she says.
She says the grandson was always being misdiagnosed with malaria because his temperature was always high, but the problem never went away.
"I was told that the heart has fallen and I used traditional medicine to treat him with no reprieve."
One day, community health workers from a Pentecostal church-related organization in Busia County visited her and identified what has been ailing the boy.
"They took me to Kisumu where we went to Aga Khan Hospital and Getrudes where they realized the heart had an issue and we were referred to Nairobi for specialized care."
She was full of joy that her grandson would finally get the much help he needed to live a normal life
"I am excited that my grandson has undergone surgery and I am sure he is going to get better and I will focus on other things. My grandson will also be able to go to school and play like other children," Clementino says.
Heart conditions vary from congenital heart defect which is a problem with the structure of the heart that a child is born with or Rheumatic heart conditions which is a critical form of acquired heart disease in children rheumatic fever, an autoimmune inflammatory reaction to a throat infection that can cause death or life long disability.
Some congenital heart defects in children are simple and don't necessarily need medical intervention. Other defects are more complex and may require several surgeries for several years.
"Most heart defects are hereditary and some with genetic defects or environmental factors like infections a mother goes through during pregnancy that can affect the baby's heart quite severely. But we can't blame or pin heart defects on anything they just happen," explains Dr Terezia Tavacova, a paediatric Cardiologist
Serious congenital heart defects are usually identified soon after birth or during the first few months of life. Symptoms include pale gray or blue lips, tongue and fingernails, rapid breathing, swelling in the legs, belly, or areas around the eyes, shortness of breath during feeding -- leading to poor weight gain and fainting during exercise or activity among others.
"One of the commonest symptoms is if the baby is not doing well, not thriving, eating properly, they are not gaining weight as they should this is the first sign then later breathing problems and lung infections that's why most babies are misdiagnosed with pneumonia," adds Dr Terezia.
Some of these heart defects include atrial septal defect where there is a hole between the upper heart chambers or atria. There is also a ventricular septal defect where a hole in the wall between the right and left lower heart chambers or ventricles is noticed.
Patent ductus arteriosus is a connection between the lung artery and the body's main artery or aorta. It's always open while a baby is growing in the womb, and typically closes a few hours after birth. But in some babies, it stays open. This causes incorrect blood flow between the two arteries.
And lastly, the total or partial anomalous pulmonary venous connection occurs when all or some of the blood vessels from the lungs or pulmonary veins attach to a wrong area or areas of the heart.
"Those with rheumatic heart conditions and problems with the valve where the blood is leaking to the left atrium always get valve replacement with a prosthesis one, but that option is not suitable for children as the valve does not grow when the child is growing so what we do we repair the valve," explains Dr Peter Plasil an Intensivist.
According to World Health Organization, Five thousand children require congenital heart surgery in Kenya each year, but only a small number receive such interventions.
Annually Kenya performs around 120-150 congenital heart operations with a similar number of congenital catheter interventions.
Open heart surgeries are quite intensive as they require pediatric cardiologists, Anesthesiologists, cardiac surgeon, cardiac nurses, intensive care nurses and perfusionists to operate the heart lung machine to be al available
Unfortunately, most cardiac operations take place in Nairobi and partly Mombasa.
Approximately only Seven hospitals in Kenya are capable of performing open heart operation of which four are private. The public ones are KUTRRH, MTRH and KNH.
Congenital and also rheumatic heart conditions are treated better when diagnosed early through pre-conception screening or neonatal screening
"Heart defects are common in every country, but the difference is how can we deal with them. In the West there are a lot of opportunities to diagnose these kids quite early even before they are born prenatally, so they are born with a known diagnosis that is much easier to treat, but here medical services are not affordable and reachable making cases of congenital heart defects misdiagnosis high." Says Terezia Tavacova a paedtriac Cardioligist
She says the only way to handle congenital heart defects is to create awareness and screening to curb cases early enough.
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